Monday, June 18, 2018

Week 252: If you would like

For those of you who lost track, two weeks ago, Papa Bear came down with summer pneumonia, which Lemon and I also subsequently contracted. I got on doxycycline and started feeling much better pretty quickly, and we started Lemon on Cayston at the same time. Both Papa Bear and I noticed after a day or two that the Cayston didn't seem to be kicking in as quickly as usual, and Lemon was still pretty sick even after 2 or 3 days, when usually Cayston has him feeling well by then. Then, on Tuesday, we got the results of his throat culture from his clinic visit back. There amongst the "endogenous flora" and Staphylococcus aureus (which he has cultured since he was an infant), was a new name: Haemophilus influenzae.
Culturing a new bacteria is never good news, so I did what any responsible person would do. I dropped the name of this new arrival into Google. Aha. A well-known respiratory pathogen that causes (you guessed it) pneumonia. So, it seems pretty clear that this is what made us all sick (risk factors: attending preschool). Given that there was a new bug, and that Lemon was still sick, I sent a message to our nurse practitioner asking whether we should do anything else. She said that their general philosophy is that they don't treat H. flu, but that we could add an oral antibiotic "if I would like." Am I missing something here? If I would like? Yes. I insist on it. My kid with a genetic predisposition to lung infections has a documented new bug that isn't responding to our usual intervention plan. I must have new alternatives immediately. No putzing around. Why on earth aren't _you_ insisting on it? 

I managed to write back something a little more diplomatic, like "Oh yes, I think it would be good to try something additional," and we started Lemon on azithromycin that day. It was definitely the right decision, he started feeling better almost immediately, and his cough is slowly drying up. We're continuing with Cayston as well, so hopefully by the time two weeks of Cayston are done, we'll be back to baseline. But seriously. If I would like.

In other healthcare annoyances, let's talk about health savings accounts. We have one. We are fortunate enough to have enough financial resources to be able to set some aside in there and reap the benefit of spending pre-tax dollars on our various healthcare things. But man they do not make it easy to spend those dollars. Take our home pharmacy for example--that's where we get all the supplies for Lemon's tube feeds. Every month, I get a bill for a random number of dollars, usually between $10 and $200, which in some way corresponds to a number of dollars that we might owe for receiving the exact same set of supplies every month. 

Let's say I want to pay that bill with our HSA. I have to call a phone number, spell my name about eight times, explain that yes I have a different last name than the patient, and then have the person there read me a number of dollars that she thinks I owe. This number bears at best a limited relationship with the number I have on the printed bill in front of me. If I ask to pay the actual number on the actual bill that I have, I am informed that my choices are to pay my balance in full, or not. I opt for in full, and then agree to have the receipt emailed, which results in my having to spell my first and last name again several times, while trying to indicate that my middle initial comes between them in my email address. Eventually, we come to an understanding of how my name is spelled, and I hang up the phone. Moments later, the receipt arrives. Hooray!
Just kidding. The HSA people then inform us that the receipt from the provider is insufficient documentation of the expenditure. Papa Bear then has to call and get an itemized statement from the pharmacy (the statement that I have at home is of course useless because its number of dollars is not the same number of dollars that I had to pay in order to have the privilege of paying with my HSA card). The new statement arrives, but is tragically destroyed when our basement flooded this weekend (no joke). Papa Bear figures out that he can download some corresponding "explanation of benefits" forms from our MyChart account, which are convenient PDFs that he can then upload to the HSA people's website. Jinx! The EOB forms that you download are 2.1MB each, whereas the maximum file size allowed by the HSA website is 2.0MB. So, each one has to be individually saved as a lower-resolution PDF before being re-uploaded to the HSA website. 
And that is what it takes to spend $102 worth of our HSA. Just a little something to keep in mind if you hear anyone touting HSAs as the solution to all that ails the healthcare system.











Anyhow, enough of that. The kids started swimming lessons and summer camp, and love both so far. I'm hoping we got our summer health crisis out of the way early, so we can just enjoy the next few months!







  

Monday, June 11, 2018

Week 251: Pill poppers

Just to set the stage for the week's events, I remind you that about 10 days ago, Papa Bear came down with a case of summer pneumonia. He may or may not have caught the underlying bug from Lime, but that aspect of the situation is lost to history at this point. What is clear is that Papa Bear was diagnosed with pneumonia, got on antibiotics, and was feeling sufficiently well on Monday morning of last week that he left for 3 days at a conference in California. I'd felt less than great on Sunday afternoon, but attributed it to maybe overdoing it on a long run, and felt OK enough Monday morning when Papa Bear left. Then began a cycle of alternately feeling fine/feeling terrible. Although I didn't map the periodicity too closely, I did notice a sort of trend where I would take 600mg of ibuprofen, feel fine for 7.5 hours, and then feel awful. I also developed a cough of sorts. 
Anyhow, by Friday, Papa Bear was back, I was still feeling lousy at 8-hour intervals, and a doctor friend of mine wisely suggested that I take myself over to urgent care. I did that, and the urgent care people thought that since I was feeling awful, had a cough, and had a recent pneumonia exposure, they were willing to go straight to antibiotics, and I'm glad to report that I'm now feeling much better, even in the absence of ibuprofen. I did everything I could to minimize Lemon's exposure to the illness, but as I'm sure you can all appreciate, parenting single-handed with a productive cough is bound to result in someone getting exposed to something. And sure enough, Lemon has it now, too. We started him on Cayston at the same time as I started my antibiotics, and although he's still coughing a lot it's clear that things are going in the right direction. 



Have I mentioned that it is June?





In other news, we got the first batch of lab results back from Lemon's check-up last week, and they look really good, pretty much everything within or near the normal range. His liver enzymes are up a smidge from last time, possibly because he's grown so much that he outgrew his ursodiol dose, so we've increased that again and will follow up with the GI doctor in the fall.

Our other big adventure this week has been the start of teaching Lemon to swallow pills. He was skeptical (of course), but I do really feel like it will make his time in kindergarten so much easier if he and his teacher aren't messing around with apple sauce at every meal. We started with this video, recommended by our dietitian: 



So far, we have been practicing first with just plain water, and then trying to swallow TicTacs. The first two times we tried the TicTacs, Lemon didn't swallow them and spat them out. But, then, tonight, miracle of miracles, he swallowed one. He couldn't believe it himself. Of course, swallowing one TicTac is far from being able to swallow 3 pills, each larger than a TicTac, before every meal, but it is a start, and honestly more progress that I would have expected us to make in just 3 days, given how Lemon is about, you know, putting things in his mouth and swallowing them. 

Tomorrow I have my orientation meeting with our summer camp, which should be exciting. I somehow suspect they may have some questions about the medical form I filled out for them...

Monday, June 4, 2018

Week 250: Poop doula

I must say, in a household with someone with CF, it is a rare week when everyone EXCEPT THE CF'er has some sort of respiratory issue. Lime had it first, a cough that has taken the better part of two weeks to more or less clear up. On Tuesday, Papa Bear was not feeling well, although he thought it was because the air conditioning in his office failed and he got dehydrated. By Thursday, with a fever of 102.6, it was pretty clear that something more serious than dehydration was going on. He decided to head over to urgent care. As he was leaving, I semi-joked, "I hope it isn't summer pneumonia." Haha, it was summer pneumonia. Because whose husband gets summer pneumonia? Mine, it seems. Luckily, he got on antibiotics right away and started feeling better almost immediately. And then, I went out for a long run on Sunday, feeling OK at the start but definitely not OK at the end, and then developed a low-grade fever and a cough too. Luckily I seem to have only gotten whatever Lime had and skipped the summer pneumonia. Meanwhile, Lemon has just been skating along, having apparently built up immunity to whatever this is sometime earlier in his preschool career.

In other exciting news, I sold our changing table this week. After almost 5 years of constant use, it is ours no more. I wish the (very) pregnant person who bought it the best of success. Now, I have moved up in the world from diaper changer extraordinaire to what Papa Bear refers to as "poop doula." I do hope that as Lime gets older, my services will be called on less frequently, but it is absolutely a step in the right direction.

We had our clinic visit today. After two appointments that were basically nightmares of waiting in the exam room for people to see us, my complaints seem to have finally registered and this appointment was perfect. We actually got there earlier than I usually do for appointments, because I had remembered it as 1pm, but it was actually supposed to start at 1:15. However, no sooner had I checked in at the kiosk than the pulmonary function testing person called us in, so we got that out of the way first with no wait at all. Lemon did the test really well, an impressive feat for someone who is not yet 5. He also managed to break the scale in the PFT room by sort of leaping off it sideways while also simultaneously twisting before his feet were actually off the platform, such that the platform came off with him and crashed to the floor. But anyhow, that is life with my kid.

Speaking of scales, Lemon now weighs 44lb and is in the 83rd percentile for BMI for his age. This is up from 9th percentile when he had his G-tube placed. This appointment was actually pretty hilarious, because the care team spent the whole time sort of asking me what I did to accomplish this miracle, and looking to me to see if I thought any further changes were needed. I think we're good for now. Our main goal for the summer is just to keep packing the weight on so that Lemon is prepared for whatever new contagions he will encounter in kindergarten.

I also asked the clinic staff what they thought about the 504/IEP issue--do we need a formal plan for the school to deal with any CF-related needs? The advice I got from the clinic was no, he doesn't need one, especially for the early grades. Contrast that to the advice I've gotten from all the parents I've talked to with kids with CF or other special needs, who have all said emphatically yes, you must have one. I think I'm going to trust lived experience on this one.

I finally finished up my most recent set of articles for the CF Foundation website, so I should be able to point you all to those shortly. I've signed up to be part of our state's advocacy team--not sure what that entails yet, but I'm hoping that I will get a chance to meet with one of our national representatives later this year. I'll keep you posted on that as details emerge.

Also, how is the last day of the school year on Friday???






Monday, May 28, 2018

Week 249: Heat wave

Not 6 weeks ago, we had snow on the ground here, and for the last 4 days, the temperature has been above 90 degrees, a record-breaking heat wave. This was a good reminder that just as there are CF things to do in the winter, there are also things in the summer. Lemon is incredibly sensitive to heat, and turns bright red in response to even a brief heat exposure. So much so, that Papa Bear thought he had gotten sunburned (not an unreasonable thing, given Lemon's near-translucent skin tone). But, sure enough, after a half-hour or so in our air-conditioned house, Lemon was back to his ghostly little self. We've started adding extra salt to Lemon's overnight feeds as well, to try to give him back some of the salt he's losing to sweat during the day.







Beyond things having gotten a little too hot too fast, both kids have been flourishing since the end of winter. It's been amazing to watch them come out of hibernation and run themselves ragged every day with constant outdoor adventures. The warm weather has been incredibly good for Lemon's appetite. Papa Bear and I were estimating that he probably is actually eating several hundred calories a day by mouth now, which is quite an achievement for him. Still not enough to sustain himself, but I do feel like for maybe the first time he is actually experiencing hunger and understanding that eating is the way to address that feeling. Having a little brother who is in basically continuous graze mode certainly helps too.






With today being Memorial Day, it is also starting to hit home for me that Lemon's time at his preschool is drawing to a close. In two short weeks, he will be done with 4K and on his way first to summer camp and then to elementary school. In other words, I need to get started on laying the groundwork for him in both places. We have a clinic visit coming up next week where I will see what guidance they can offer me in terms of getting things organized for him at the elementary school, and I should really reach out to the summer camp soon to deal with training someone there how to give him his enzymes, and get whatever doctor's note they need in place.








I'm also pretty optimistic that we will be able to teach Lemon to swallow pills sometime between now and September. His gag reflex is way better than it was a year ago, and food school has given him a lot more confidence in his oral skills. I'm thinking that doing enzymes at school will be easier and less obtrusive if they don't have to get involved in the whole capsule opening/applesauce scooping scenario. So, now our main challenge is just finding the time to work with him on this new skill every day. Hopefully we'll be more successful with pill swallowing than learning to read...






Monday, May 21, 2018

Week 248: Walk on

First off, a tremendous thank you to everyone who donated to our Great Strides campaign this year. Thanks to a late surge in donations, we raised almost $5000 to support the incredibly important work of the CF Foundation. This coming year is going to be big one for CF research, and it means so much to us to have your help in supporting and accelerating these efforts.

The walk itself was fun as always, since it is one of the few chances we get every year to connect with other CF families. As with years past, we left Lemon at home. Some parents do bring kids with CF to the walk, but we haven't yet. Lemon is just too wild, and too interested in other children, so there is no way we could ensure his safety and the safety of the other CFer's at the walk, from an infection control perspective. Maybe someday he will be sufficiently domesticated to bring along, but I'm not holding my breath!

I'm actually writing this post from a hotel room in Minneapolis, so I am not set up to post any photos this week and I will be keeping it brief.  On my drive to Minneapolis, I stopped through Rochester to see an old friend. He has children with Type 1 diabetes, which is obviously very different from CF. Yet, it was incredible to see how much of the experience of parenting children with a chronic health is the same regardless of the exact condition. The endless battles with insurance companies, the prescriptions that are from multiple pharmacies and never synchronized, the challenges dealing with schools, and worries about the future. Surely there must be a better way to do all of these things?

I also feel like there may be a missed opportunity in terms of connecting with parents of kids with other health issues. I've built a nice network of support through in the CF community, but the community is pretty small and limited by the fact that we can't really get together in person. There are lots of organizations out there, but (as far as I know) they are each focused on one disease. In this connected age, it should be possible for us to link up somehow...

Monday, May 14, 2018

Week 247: Minutes of fame

I believe I have now used up 3 of my allocated 15 minutes of fame. This week, I appeared on our local NBC affiliate to talk about cystic fibrosis and Great Strides. The development director for the local chapter of the CF foundation had reached out to the station months ago, and the anchor of the 4pm news show agreed to have us on for a short segment to publicize the walk. The original idea was that we were going to have 4 minutes, and both the development director and I would appear. Then, the development director had a scheduling conflict that she couldn't get out of, so it was just going to be me. Then, news happened, so the time slot got cut down to only 2.5 minutes, so it was probably just as well that only one of us appeared on the show.

I got to the station about 15 minutes before 4 and was ushered in to the studio. The only mental image I had of a TV studio was the glimpses I'd caught on the rare occasions that I'd watched a live show in the past. Those mental images included lots of people running around, manually pointing cameras and lights. Maybe that's how some bigger studios still do it, I don't know. What I can tell you for sure is that NBC15 in Madison doesn't do it that way. The total list of people on the set for the 4pm news in Madison was myself, the anchor, and the weather guy. There were many cameras and lights, and they did move, but they were all controlled remotely from a big room down the hall.

The anchor was really nice and chatted with me before the show started and through all the commercial breaks. It was pretty hilarious to hear her transition from regular lady-next-door voice to serious news anchor voice and back again from sentence to sentence. Secret information: the weather guy is wearing a jacket and tie, but also sneakers since you can never see his feet. I sat on the interview couch while the rest of the news went on, and then a member of the crew came in and wheeled the couch into position in front of the cameras right before my segment.

You can view the segment here: http://www.nbc15.com/video?vid=482330961




I had practiced what I thought I was going to say, but the anchor hadn't told me exactly what questions she was planning to ask me, so I was caught off guard by a couple of them. And then, just as I felt that I was sort of hitting my stride, the segment was over. At least three different people checked to make sure I'd turned in my microphone, and I went home. Hopefully the segment connected with a few viewers who wouldn't otherwise have given CF or Great Strides a thought, but it's impossible to tell--there's even less feedback than with blogging, where at least I can see how many people looked at the blog each week!

Speaking of feedback, how about heading over to our Great Strides page and making a donation? The walk is this coming Saturday May 19, and we are about half-way to our team goal. At the moment, Papa Bear is ahead of me on fundraising, which is sort of embarrassing (in a good way). So, head on over by clicking here! I'm trying to follow NPR's new model of having the fund drives be really short, so let's make it sweet. Thanks in advance for your support--there is no cause more important to us!


Monday, May 7, 2018

Week 246: Getting oriented

Saturday was Lemon's kindergarten orientation. I was a little apprehensive about it, because he can be pretty shy, but it turned out he was more than ready for it. He was so excited to walk from our house to the elementary school like a big kid, and went off with his teachers and a bunch of kids he didn't know pretty willingly. It helped that there were two kids from his preschool in his orientation group, but he got caught up in the action so quickly that I'm not sure how much it mattered.

His teachers and I had discussed how to deal with snack time, since he would need to take his enzymes before eating. Ultimately, we agreed that I would just slip into the classroom at the appointed hour to give the enzymes myself, since the school needs a doctor's note in order to give medication (the fact that his enzymes are actually prescribed by a nurse will, I assume, be a battle for yet another day). I was a little concerned that he would want to leave with me after getting his enzymes, but that worry was also misplaced, since he was so engrossed in the story that the teacher was reading that I could barely get him to turn his head towards me long enough to actually take the enzymes. So, all that seems promising, and he was very disappointed this when I told him that he had to go to his regular school this morning rather than back to kindergarten.

The kindergarten orientation did raise another issue in my mind, which is whether or not to file a 504 plan for Lemon. A 504 plan is a plan for the school to accommodate students with permanent disabilities. There is no doubt that CF is a qualifying disability, although I never ever in my own mind think of Lemon as disabled. Nonetheless, there are some accommodations that would probably make school easier for him. Another mom that I talked to (whose son has other issues, not CF) said that in her view the 504 was the best communication tool to get the staff of the school on the same page about what the child needs. That way of thinking about it resonated with me more so than thinking of it as a disability plan. So, I guess one project for this summer will be figuring out how to get such a plan in place. 

Lime is continuing to do pretty well with potty training. He was having a hard time doing #2 on the potty, though. It's a very common phenomenon among toddlers, apparently. One bad #2 experience and they resist doing #2, which inevitably results in subsequent bad #2 experiences and away you go down a #2 spiral. I am quite sure that if Lime had been my first kid, I would have been trying all kinds of things involving prunes, bran cereal, copious amounts of water, and endless cajoling/praising/bargaining. But, guess what, Lime is my second kid and my first kid has CF and has been on a staggering array of medications since he was 10 days old. So, I looked at Lime's situation and thought, I bet there's a chemical that can help with that. Sure enough, there is, and it comes in watermelon-flavored tablets that Lime finds quite delicious. And thus, we broke the #2 spiral. Better living through chemistry, people.

Speaking of better living through chemistry, let's talk again about drugs that treat the root cause of CF. Right now there are three on the market--up from zero when Lemon was born. Unfortunately, none of the drugs that are available now will
work with the combination of mutations that Lemon has. But, good news: medications that will work for him are in Phase III clinical trials, meaning they could be approved in the next couple of years for adults, and a couple of years after that for kids. We are so close to something that could fundamentally change Lemon's life trajectory, but we need to complete these critical trials to know for sure that these medicines will work well. Donations in any amount will help make sure these medicines are ready for Lemon to take, hopefully before he finishes elementary school. We are so close--help us get to the finish line! Please click here to visit our team page and help us achieve our fund-raising goal before our walk on  May 19!